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Journal of Child Neurology
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Infantile Alexander's Disease: Serial Neuroradiologic Findings

Qing Ni

Department of Neurology

Gretchen S. Johns

Department of Pathology

Anantha Manepalli

Department of Pathology

David S. Martin

Department of Radiology

Thomas J. Geller

Department of Neurology Saint Louis University Hospital Saint Louis, Missouri

Serial neuroimaging studies in Alexander's disease were obtained on an African-American girl who died at 4z\x years of age. She presented with macrocephaly, psychomotor retardation, spasticity, a seizure disorder, and hydrocephalus. A thorough metabolic evaluation of defined leukodystrophies, including Krabbe's disease, adrenoleukodystrophy, metachromatic leukodystrophy, Canavan's disease, and Leigh disease, was negative. A diagnosis of Alexander's disease was made based on the clinical features and ruling out all other possible causes. It was confirmed by pathologic findings of numerous subpial, subependymal, and perivascular Rosenthal fibers throughout the entire cerebrum. Interestingly, autopsy also identified the stenotic sylvian aqueduct owing to Rosenthal fiber accumulation, explaining the origin of hydrocephalus. The evolution of magnetic resonance imaging findings appears to be unique in this disease. (J Child Neurol 2002;17:463-466).

Journal of Child Neurology, Vol. 17, No. 6, 463-466 (2002)
DOI: 10.1177/088307380201700615
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This article has been cited by other articles:


Home page
 J Child NeurolHome page
E. Franzoni, M. S. Van der Knaap, A. Errani, M. C. Colonnelli, R. Bracceschi, E. Malaspina, F. C. Moscano, C. Garone, J. Sarajlija, R. A. Zimmerman, et al.
Unusual Diagnosis in a Child Suffering From Juvenile Alexander Disease: Clinical and Imaging Report
J Child Neurol, December 1, 2006; 21(12): 1075 - 1080.
[Abstract] [PDF]


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